Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038362 (stomatitis)
 8,852 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dystrophic epidermolysis bullosa (DEB) is an inherited blistering skin disorder caused by mutations in the type VII collagen gene (COL7A1). Therapeutic introduction of COL7A1 into skin cells holds significant promise for the treatment of DEB. The purpose of this study was to establish an efficient retroviral transfer method for COL7A1 into DEB epidermal keratinocytes and dermal fibroblasts, and to determine which gene-transferred cells can most efficiently express collagen VII in the skin. We demonstrated that gene transfer using a combination of G protein of vesicular stomatitis virus-pseudotyped retroviral vector and retronectin introduced COL7A1 into keratinocytes and fibroblasts from a DEB patient with the lack of COL7A1 expression. Real-time polymerase chain reaction analysis of the normal human skin demonstrated that the quantity of COL7A1 expression in the epidermis was significantly higher than that in the dermis. Subsequently, we have produced skin grafts with the gene-transferred or untreated DEB keratinocytes and fibroblasts, and have transplanted them into nude rats. Interestingly, the series of skin graft experiments showed that the gene-transferred fibroblasts supplied higher amount of collagen VII to the new dermal-epidermal junction than the gene-transferred keratinocytes. An ultrastructural study revealed that collagen VII from gene-transferred cells formed proper anchoring fibrils. These results suggest that fibroblasts may be a better gene therapy target of DEB treatment than keratinocytes.
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PMID:Fibroblasts show more potential as target cells than keratinocytes in COL7A1 gene therapy of dystrophic epidermolysis bullosa. 1654 Oct 96

The distribution of epithelial E-cadherin, basement membrane type VII collagen, and underlying connective tissues fibronectin were investigated immunohistochemically and compared in normal palatal mucosa and in denture-related stomatitis (DRS) derivatives using monoclonal antibodies.Biopsies of palatal mucosa were obtained from twelve patients enrolled in this study, 8 with type II DRS and 4 with healthy mucosa.Our findings bring to the fore, using the expression of three components (E-cadherin, collagen type VII, fibronectin), the continuities of the disorder among epithelial, basement membrane and connective tissue in the case of DRS. In type II denture-related stomatitis, we found an expression of E-cadherinin all the strata of epithelia, and the diffuse and strong expression of type VII collagen at the interface between connective tissue and epithelial cells with discontinuities in BM. The strong expression of fibronectin in underlying connective tissue with penetration in some areas of the palatal mucosa may be an early consequence of advanced DRS. Nevertheless; no single change is pathognomonic of this inflammatory process.In normal tissues (healthy clinical aspect), E-cadherin was found to be restricted to the upper strata of the epithelia, and type VII collagen revealed thin linear staining in the basement membrane and fibronectin in underlying connective tissue combined epithelia.In the case of denture-related stomatitis DRS, these three markers reflect the immunohistological modifications from the superficial layer of the epithelium to the lamina propria.
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PMID:Distribution Patterns of E-Cadherin, Type VII Collagen and Fibronectin in Denture-Related Stomatitis: A Preliminary Study. 2229 62